Background Granulomatosis with polyangiitis (GPA) has been transformed from life-threatening conditions to chronic relapsing long-term diseases as a result of significant advances in immunosuppressive therapy. Structured clinical assessment using Vasculitis Damage Index (DVI) should form the basis of a treatment plan and be used to document progress.

Objectives To investigate the Vasculitis Damage Index and clinical manifestations in localized and systemic granulomatosis with poliangiitis in Mexican patients

Methods We enrolled 61 patients with GPA according to The American College of Rheumatology (ACR) criteria at a referral hospital during the period from 2005 to 2015. Clinical and laboratory data, organ involvement and the Vasculitis Damage Index (VDI) were recorded at baseline. Patients were divide into systemic and localized form for their analysis.

Results They were 61 GPA (34 men and 27 women) mean age 42 years old at diagnosis. Systemic form was observed in 53% and localized form 47%. Chronic sinusitis was the most frequent manifestation in 33% followed by otologic in 26%. Subglottic stenosis 4 patients, alveolar hemorrhage 1%. Of the patients with the systemic form 22 presented focal and segmental glomerulonephritis and 10 patients (32%) rapidly progressive glomerulonephritis. Distal-symmetric polyneuropathy and cranial neuropathy were present in 24%; scleritis 24.5% and proptosis in 18%, palpable purpura 26.2% and ulcers in 9 patients (14.8%). The VDI score in the systemic form was 3.8 and in the localized 2.6, p= NS. The disease related damage was pronounced in kidneys and upper airways. The majority of patients in the induction to remission phase received steroids plus cyclophosphamide, 7 patients also received plasmapheresis and in maintenance phase they were treated with methotrexate or azathioprine.

Conclusions In this cohort of patients with GPA, a high chronic damage was found which was similar in both systemic and localized forms of this vasculitis. The VDI was more prominent in kidneys and upper airways in GPA patients

References

1. Bhamra K, et al. Damage assessment in ANCA-associated vasculitis. Curr Rheumatol Rep. 2012; 14:494–50.

2. Kamali S, et al. Predictors of damage and survival in patients with Wegener's granulomatosis: analysis of 50 patients, J Rheumatol 2010;37:374–78.

3. Luqmani R, et al. State of the art in the treatment of systemic vasculitides. Front Immunol 2014;5:471.

References

Disclosure of Interest None declared